● Also called transient abnormal myelopoiesis, transient leukemia
● Affects 10% of newborns with Down’s syndrome
● Resembles congenital acute leukemia; occurs within first days of life with numerous blasts in peripheral blood, more than those in marrow
● High rate of spontaneous resolution; usually resolves in 2-14 weeks in neonates, but 20-30% progress to AML-M7 [FAB] (acute megakaryoblastic leukemia) within 3 years
● Early death in 17%, is associated with high WBC at diagnosis, increased bilirubin and liver enzymes, failure to normalize WBC (Blood 2006;107:4606)
● Rarely occurs with trisomy 21 but without Down’s syndrome (Arch Dis Child Fetal Neonatal Ed 1998;79:F215)
● Associated with hepatic fibrosis (J Clin Pathol 1995;48:973)

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